Pulmonary Hypertension Associated With Interstitial Lung Diseases

Interstitial lung disease (ILD) is a term encompassing a wide array of pulmonary conditions characterized by inflammation and fibrosis of the pulmonary parenchyma. Pulmonary hypertension (PH) is frequently encountered in patients with fibrotic ILDs and poses unique difficulties for both diagnosis and management. Patients with ILD-associated pulmonary hypertension (ILD-PH) are complex, often ailing and presenting with multiple comorbidities whose individual contributions to the underlying PH can be challenging to disentangle. Evidence supporting treatment with PH-specific medications in ILD-PH is limited. This edition of “How I Do It” presents a longitudinal case-based discussion of ILD-PH to address these challenges, highlight pearls and pitfalls in the diagnostic workup of these patients, and provide a framework for the practical evidence-based approach to accurate diagnosis and management of these challenging cases.